Just doin’ our thing.

We are still here, just cruisin along, doing what we do. Getting through each day, trying to soak up and enjoy the most out of each day.

for the most part – this means we get up, do the school craziness, i do what I can on the kendall craziness, we play playdoh, sissies come home from school, and we do the dance craziness. (notice a theme?)  we still don’t have full nursing help, which is both a blessing and a very hard thing to deal with some days. i am LOVING spending time with just kendall every day. She is loving it too. we play a lot of playdoh. Read a lot of books. Watch a LOT of princess movies! And i try to soak all this up. Try to hold on to how precious this time is. Try to not beat myself up for not having the girls’ rooms cleaned or the floors swept or the laundry caught up.

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I have been trying to find time to sit and write this post for three days now. In that time, Kendall has gone from “totally fabulous!!!” to “i’m a little nervous about all these crazy things she’s doing”. It’s nothing huge or major, or really anything that I can put my finger on for sure. It’s just “a wobble”. A teeny little variation off her baseline. Wobbles that she was doing prior to her last two crashes. i’m trying not to compare apples to oranges, and read too much into things. On the flip side of that – I’m scared to death that ignoring her small little warning signs will lead to another crash.  I’m so grateful that today we had our nurse who knows kendall’s baseline and could see for herself that kendall was definitely ‘off’ today. I hate trying to make all these decisions just in my own head. It’s so much pressure. And the consequences of choosing wrong are so huge.
But we have SO much fun stuff coming up this weekend, and daddy is all the way in California, and really, it’s not like she’s doing anything THAT out of the norm. She’s nauseated, has a nearly non-stop headache, and a few wonky labs. No fever, great blood pressures, heart rates well within Kendall’s normal range. Hoping that it is just this crazy heat and her body’s inability to compensate well for it.
Tomorrow it is supposed to be hot still, but storms in the evening should cool it off. I’m very hopeful that this equates to a turn back to normal for little miss. I don’t function well when every minute of my day is spent being a non-stop diagnostic calculator for Kendall. Nothing else gets done. I try to not micromanage her issues. I try to keep things moving here in our house. I feel like I rarely succeed at anything. Except that at the end of every day, I can collapse into bed and know that at least for that day, I succeeded in keeping us all alive. Sometimes that is as good as it gets.

but enough of that.

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we have one more day of therapy (wednesday) – and in a side note, that is every day for the past week! ugh! – and then thursday the Special Spaces Chicagoland team comes to take over our house and do Kendall’s room makeover! Squeeeee!!!!!! We will have to be out of the house from Thursday afternoon until the big reveal on Friday evening. YES there will be tons of pictures and YES I will post them here as soon as I possibly can!
Saturday will be spent putting all the girls’ clothes back in their closet and moving medical supplies into Kendall’s new space, and then sunday is the start of Mitochondrial Disease Awareness week, and is kicked off with our chicago “energy for life” walk. I had had a goal to have 50 people on team “Kendall Krew” (it is our first year having a team at the walk!) – and I think we might be getting pretty close to that!
and then after ALL THAT excitement this weekend, we get to spend Monday and Tuesday in Milwaukee at CHW for appointments and Kendall’s monthly IVIG infusion.  Oh my goodness I’m exhausted just thinking about it all! But SO glad we get to do it all!

This is turning into just another boring update and i’m falling asleep typing it. mornings come WAY too early after a night spent checking alarms and giving meds.
As usual – I have so much I want to say/type/blog about – but the tiredness is making it hard to form coherent thoughts. So at least this is an update of what is going on – and hopefully the other things will get a chance to come out soon enough.
I’m so thankful for all of you who come here to check on things even when its been weeks since my last update! Please do’nt stop doing that. Some day I will get in a good rhythm and have lots of fun randomness for you! I might even have more crazy videos or horrible drawings for you soon!

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ok i’m still rambling.

have a good {fill in the blank with whatever time of day it is for you}.

love and hugs~

 

T-crest.

He Speaks. Again.

So it’s that Time of the Year again.

So Mito awareness week has come and gone. A couple of weeks ago Terra again asked me to write a blog post for Mito week and once again I have waited for the week to end before writing. Oh well, does it really matter? Realistically it’s Mito Week every week at our house.

Last year Terra asked me to write a paragraph about what Mito meant to me and it turned into about twenty paragraphs. I think Terra about fainted when she found out that I had that much junk rolling around in my head. So this year I will do this same. It will probably be shorter and maybe just an update from my post last year (I’m sure Terra will link last year’s post in here somewhere).

Not much has changed on my thoughts about Mito. I hate it and it still sucks!!! Ok, thanks for reading thisIMG_4547 post is now over……….

well not really.

Like Terra does on occasion in her posts, I ramble along once I actually get started. I still do not like to talk. I still do not like to share my opinions outside of the home or work. But I will again try to open up a little bit this year.

In my non-medically minded brain Kendall has had a good past year. In my memory-challenged mind, I believe it had been about a year since Kendall had been hospitalized that actually kept her inpatient overnight. This lack of hospitalization can be credited to many things but I feel it is mostly due to Terra’s oversight and monitoring of everything Kendall and the TPN nutrition she has been getting for over a year. It is not fun hooking up an IV to her central line every single night that runs for about 16 hours a day but if it keeps her home we will do it. This isn’t to say that everything has been great with Kendall medically. She had been having increasing issues with her bladder and has lost function in her bladder like she has in her gut. I don’t want to rehash all her medical stuff but this is how Mito works. Every time she gets sick her baseline drops, she loses function and so far with Kendall that function isn’t coming back. Hence the most recent round of hospital stays due to bladder surgery and infections associated with them. She now has another “port” into her bladder that we can drain urine out.

That’s the Ben Atkinson Cliffs Notes version of Kendall medically over the past year. If you want more, read all of Terra’s blog archives. But most of you aren’t reading this (my post) for my medical update of Kendall, it’s my perspective on the whole situation.

My perspective on the medical situation is easy. I make decisions based on logic without emotion. Terra makes decisions based on how she feels and her emotions. This is how Terra and I are different on the medical side of this Mito junk. I also feel that this difference between Terra and I is very helpful when making decisions for Kendall. For example, take the recent bladder surgery. My question to answer, although painful, was very simple, would the surgery improve Kendall’s quality of life? That is it -that is all I needed to ask. Terra had so many more questions to answer. Terra thought about the pain level, the surgery itself, the risks, the scars that Kendall would have, the additional tube, the recovery and I’m sure many more. But Terra and I balance each other out in the decision making process because we get to see each others thought process and views and make what we feel is the best decision for Kendall medically and I guess socially.

Let’s move away from the medical stuff and into what you all really want to see and read…the personal crap.DSC_5348 The stuff that goes on behind the scenes in my mind and how I see our family. I can only assume some of these things are typical of living life in a house with a medically fragile child. This is what I hate about Mito and I guess any other disease that affects families. First off, yes Kendall is medically fragile. Most people get to see Kendall when she is at church smiling and looking fairly normal. Realistically we don’t take her out much when things are acting up inside of her. Why would we? Here is what I see in my mind when I think of Kendall being medically fragile. Picture beautiful Kendall smiling ear-to-ear as she does standing on top of a large red rubber ball, balancing her now three drainage bags draining her stomach, gut and bladder in her hand and her TPN backpack on hooked up to her IV while slowly rolling that red ball on the ledge of a fence where on one side of the fence is a 500 hundred foot cliff that drops into the Ocean and on the other side of the fence is only a 250 foot cliff. When she losses her balance she falls either 250 or 500 feet. Which one would you pick? They both suck and lead to nothing good. This is why we take Kendall out on a very limited basis but it is getting even harder.

Kendall is continuing to develop and becoming so much more like a three, almost four year old. This is awesome but so challenging at the same time. Kendall gets “left out” too often when we have plans as a family. She is now very conscious of when we leave and she is left home with her nurse. She hates being left out and it kills me when we have to leave her. We constantly have to make family decisions based on how Kendall will be affected. Will she miss a much needed nap, is it too hot, is it too cold, is there a small chance she will get wet and ruin one of her many tubes sticking out of her body, does she have attachments connected to one or all of her tubes, will we be out too late and past her 7pm bed time, is she losing her balance on that red ball and teetering ever so close to falling off of that cliff. All of these questions come into play when we try to do simple everyday items. How can we continue to live like this?

Here is the thing; I think we strive to live through these kinds of decisions and day-to-day medical fragileness, and have been doing a decent job of it, but at some point, the juggling act that Terra and I do is bound to fall apart. This is the hard part to put down on this post. As I said last year, Terra is one of the strongest people I know and I am sure several of you out there wish you had her strength, I know I wish I did. I still feel the same way. Terra is so very strong. But are my thoughts of Terra being so strong the wrong way to think about my wife? I try to support her but it’s not something that I have done very well. As a matter of fact I think I fail at this consistently. As I reflect on this now the way of support that I provide is probably too often not what she needs.

I support Terra through exhaustion myself. With my work schedule I travel 40-45 weeks a year, being gone on average three nights during the week. Many have asked why I don’t get a job where I stay home and don’t travel regularly. Reality is that I have so much freedom in my schedule that I can drop everything and rush home if an emergency comes up. I don’t know if I would have that ability if I did something different, at least that is what I am currently telling myself. I work long hours during the week (as many do) and then come home and get more exhausted. I do my best to support Terra by taking over the care for the kids so she can get a break. Actually while I type this Terra is at a hotel at an undisclosed location in the Chicago area because this week and the last few were just too much. I try to support Terra by sending her away??? Does that make any sense to people out there? Maybe not when my focus on support is by giving her time away? Don’t get me wrong I know that she needs the time away, heck I think every mother needs some quiet time away. They probably need more time away then we (fathers/husbands) give them. But Terra also heads out a lot with her friends to just talk to a real person instead of a computer like she does during the week. Are you seeing a pattern here? Why am I sending here away all of the time? I definitely think the time away for her is very healthy but what am I not giving here. I am not giving here my time!!! Ding, Ding, Ding I am the winner of my own question contest. This is again why I hate Mito. It is not the disease itself but the aftermath of what I think I have become and how this affects my family. I have consumed myself into my own little shell and am not supporting Terra in a way that she needs.

So why am I going into my shell? Please don’t take this wrong but I am so sick and tired of talking about Kendall’s Mito issues. This is all we ever talk about. I have become bitter and hard and apathetic. This sounds strangely familiar to last my post last year but I think it has become worse. I hate that I have become these things.

I continue to be apathetic towards my faith. When I can I go to church just to sit there and wait for it to be over to pick up the kids. I saw a picture on Facebook this morning that rang true. It stated something like this “Going to Church makes you a Christian as much as standing in your garage makes you a car.” Wow, I have to think about that some more. Our church is awesome, the support that they give us in genuine and in my opinion rarely found out there in the world of churches. I say I am still angry at God but now I just don’t know if its anger at God or disappointment at myself.

My heart continues to break for the older three girls also. The “normal” childhood for them is just not a reality. They just don’t get to do normal childhood things. I think Kealey knows that Kendall may someday not come home from the hospital, she has actually probably known for some time. She is a smart one, she is. How is this a way to live, knowing this kind of reality? Well it isn’t. Terra and I have always said that we don’t want Kendall’s issue to dictate our lives. We try this but reality is Mito dictates our lives, dictates the girls lives. They get the brunt of an exhausted mother during the week and an exhausted mother and father on the weekend.

This exhaustion also has an effect on the relationship between Terra and I. I get exhausted, zone out and go to sleep. Terra gets exhausted and dives into her support system on the internet. I am so done with talking about Mito issues that I am done talking or being present when Terra needs me. I don’t know when we, or more I, started using this as an excuse to stop being there mentally. Obviously we cannot just stop talking about Mito issues. They rule our lives right now. I must get better at supporting Terra in a way that she needs it. I will never replace the support group from the internet but I need to become the husband that she can turn to for support.

So, for all of you that read through this whole thing I am sure you are done with reading my complaining. But reality is what it is. Living day-to-day with four young kids trying to raise them right, traveling for work, trying to support Terra in ways that she needs and a thousand other things is completely exhausting. Throwing Mito issues into the mix is getting to the point of being overwhelming.

 

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Things are not all doom and gloom. We are all still here. Kendall is still here. She blows my mind almost daily with how strong she is and how she can still flash that amazing smile amongst all the pain. Keep on fighting Kendall Quinn, you are a miracle. When your Mito issues rise up keep on fighter waive your fist in the air and yell I got this daddy!!

Prayers are always welcome; Lord knows that we need them. I need them and He needs them from me.

 

Editors note: Signing out for Benj. I think it would go something like his old Fedex job where he had to run the radio.

 

This is Ben. Copy.

Friday Fabulousness.

Again, just a day late! There seems to be a running theme this week…

Anywhooooo.

Friday was both fabulous and not-so-fabulous. I started out the morning with a raging migraine, and Kaylen started out the day just raging. Which made for a kind of crazy combo. Our nurse also decided to just rearrange her schedule in her own head, so she come flying in at 8:40, proclaims to me ‘I’m early for once, yay!!!", and then proceeds to act as if she wasn’t actually 40 minutes late. I know she was late because she was supposed to work four hours at our house and leave at NOON…but she just made everyone else fit into her schedule like it was no big thing. I’m only venting about this now because the nursing situation has been driving me even more up a wall than normal. I am not sure how it appears to people who don’t need or use nurses inside their homes to help with their medically complex kids. Like maybe I shouldn’t complain? I try not to. We have had some amazingly awesome nurses and are very lucky to be able to have the extra help with Kendall and the ability to keep her “healthy” by not schlepping her everywhere the rest of us need to schlep to, allowing her body to not expend energy it doesn’t have by running errands in the 100+degree days, having someone help us monitor all the crazy things her body does so we know when to take action and when to let it run its course. And truly, I’m not complaining about this nurse. She did a great job with kendall, for the most part. But sometimes, when you have a personality conflict with someone, it becomes very hard to have that person in your home all day every day. Especially when both personalities are quite strong, and neither is going to cave to the other.

I won’t get into a whole lot of details – but I’ll say that there have been some challenging moments over the past few weeks. It’s not a bad thing – people just sometimes aren’t meant to work together in close quarters. Not every nurse fits with every family. I have ALWAYS tried my hardest to make sure that I am not just cancelling a nurse based on some initial quirks (theirs or mine!) that can be resolved with compromise. But when things start coming into play that involve taking things out of my home, using a large quantity of things that are ours, and/or –ESPECIALLY THIS – compromising the care of our child, then it’s time to move on. This has been such an emotionally draining past few days for me. And I know that the vagueness of this is making it a hard story to follow, but I need to get it out somewhere. This is what is going on with me right now, so it’s going in the blog right now. I hate hate hate feeling like i’ve caused someone undue pain or stress. I know I talk a big game about kicking baby doclings in the shins and such – but really, having to “fire” someone from our case has never been easy. I put up with a LOT of stuff, and i’ve had this confirmed from our nursing case manager – i’ve put up with a LOT in this instance. Still, I can’t help but feel more than a little horrible about the whole situation. I wish her the best of luck finding a position that can suit her and her skill set. She CAN be a good nurse for someone. I hope she is able to find placement elsewhere soon.

And there, that’s about all I have to say about that.

It’s going to be a crazy week, or possibly two weeks, until we get some more coverage in. But we are blessed that it is “do-able”. I don’t think it’s easy to explain how a kid who “looks so good” as Kendall can require so much IMG_4500 help to stay that way. Unless you have watched her go into and back out of a crazy “cycle” with your own eyes, you just don’t get it. Every one of our nurses has had to have their “trial by fire” of thinking why does this kid need help? And right on cue, Kendall will prove it. But right now she is in a pretty good upswing, so i’m hopeful that the next couple weeks won’t be TOO crazy. Although given that it just took me almost 2 hours to give her a bath and change all her dressings/get her meds done for the morning….yeah. it’s gonna be a long couple of weeks. But we’ll be ok. I know we are actually very lucky that Kendall is doing as well as she is, and I’m not complaining about that.  Hopefully Kendall pushes through some of the “craziness” her body is trying to throw, and it doesn’t turn into anything we have to actually worry about. It’s just been one of those weeks I guess.

A week where having friends who can cheer you up with a crazy text, or a bright purple sparkly sweatshirt for your kid, or just laughing about crazy TV shows together, can make all the difference in the world. It was overall a really good week. I had an awesome dinner and talk with a good friend (who also happens to blog and whose name is Tara – we’re a match made in heaven I tell ya!), had Ben home to help with the girls all week and had a lot of fun watching stupid movies with him every night, and just got a few procrastinationy things checked off my list. (If you don’t know what procrastinationy means, then you are obviously not a procrastinator. Congratulations to you and here’s your gold star of overachieverness.)

So onward we trek.

Sunday starts Mito Awareness Week. You may remember this from last year, because it was the first time Ben blogged here on Terra Talking. I’m trying to convince him to do it this year too. (He might need a little encouragement from you all to get there – leave a comment here or on the blog facebook page to tell him you’d love to hear this thoughts again!) We will be kicking it off by attending the Chicago area Energy for 2011 Awareness Week Facebook 2 Life walk to raise awareness. Hopefully Kendall will behave for the whole thing as I will be there as “official photographer”! We don’t have an official team this year (again) – but maybe next year I will have my act together so we can walk with others who support us on this journey, as so many of you do. I asked on the facebook page for any questions you had regarding mito that I can answer this week – and i’ll ask the same thing here. You can, again, leave it here in a comment or on the facebook page. I’d LOVE to hear from you – no question is too stupid or out of bounds. I may not have answers for all of them, but let me know what you want to hear more about!

I hope you all had Fabulous Fridays too! And that you all have a wonderful weekend! Thanks for being a part of what puts a smile on my face every day – just by being you, by being here, by reading along and letting me know you’re there!

The Strong Silent Type.

I need a serious flashing blinking warning of some kind on this post.

Not only will you need a kleenex or ten, but you will need some smelling salts or some other way of reviving yourself after the shock. Today I have a guest poster. He’s the strong silent type, and I can honestly say that except for our wedding vows I don’t think I have ever heard THIS MUCH out of him at once in my entire life.

This was supposed to be part of mito awareness week – but, for this much goodness, I’m willing to overlook a few days. I am going to backdate this so that it shows up in the right place in the archives – but if you want to share it (and i hope you do), you can click on any of the little social media icons on the bottom of this post, or copy and paste the address in your address bar for this specific post.

Anyways – Ben does a good job of intro’ing his own post, so I’ll stop talking now and let you hear it straight from him. But seriously on the kleenex…go get them now.

 

Hello all in Terra’s blog/web world.

I am Ben Atkinson and yes, I do actually exist. I am not an apparition. Occasionally Terra will mention me in her blog but rarely do you all ever see or hear from me. That is just who I am. I am a behind the scenes, hide in the corner, sit in the back row kind of person who rarely speaks but likes to think when I do, people listen. Also, I have not been blessed with the gift of grammar as Terra has. My thoughts are usually short and to the point, very efficient and conclusive. I have very strong opinions on most things but rarely voice them to others because it would not be efficient to argue and I just don’t like to talk. I will attempt to alter these aspects of my personality here today and expand on what I think.

Terra has been asking me for a couple of weeks to write a paragraph for her blog about what Mito means to me. Well I am finally getting around to doing this but am sure it will be longer than one paragraph. I can only hope that it will be in some form of coherent thought.

What does Mito mean to me? It sucks, I hate it! I would use more foul language to voice my hate but I know my in-laws would not approve and are probably reading this. How much do I hate Mito? I hate Mito with all the passion that burns inside of me.

Now I need to make this next point very clear and keep this in mind as I write the remainder of this. I do not hate Kendall, I do not blame Kendall. These issues that I talk about are not Kendall issues. These are Mito issues. I hate Mito issues. Kendall Quinn is a shining light of strength and joy that I have never witnessed in another human being. I do not have very many people in this world that I would consider to be a hero, Kendall is on the top of a very short list. I simply hate Mito and the affect that it has had on my family’s life. So my anger may be better directed to the results of Mito rather than the actual condition itself. However, I have some control over those Mito results and how it affects me and my family so maybe I will blame the disease and take no ownership of the following frustrations with Mito. But I will own up to them.

What does Mito mean to me? That question keeps running around in my mind and reality is I have been doing some soul searching when it comes to what this question means. What I say over the next few paragraphs may be a surprise to probably all of you including very close family members. But it is the reality of what I have been thinking over the past few years. It is thoughts that I have hidden away in my own inner core of my being because along with my lack of wanting to talk to people I also tend to hide away my own feelings from just about everyone who I interact with. You can image the challenge this posses for Terra who has to deal with me on a continual basis.

So the affects of Mito have caused me to question and wrestle with many differing emotions. Many of these feelings stem towards my ability to be selfish through all of this. Why has this had to affect me, why has my life needed to be flipped end-over-end? I have become bitter, some times more than others. I have been angry, again, sometimes more than others. However I am thankful that both are subsiding.

My bitterness and anger has been directed at so many different focus points. My bitterness runs deep because as I stated earlier I hide my emotions and hold on tight. I have been bitter and downright angry at God. “Why has he done this to me?” I have questioned many times. Yet at the same time I am questioning him I am not reaching out to him. I am not letting him carry me through these difficult times. I have always been the person that could keep it together. I stood under my own power and never needed anyone else. Unfortunately by doing this I have alienated many in my life. I still struggle with this anger and bitterness but it is slowly subsiding. Again because of my own self inflicted need to keep my emotions to myself I was only able to tell Terra of this about a month ago. What is funny (not in a laugh haha kind of way but more of a twisted what am I thinking kind of way) to me now is that so many times God has pointed out to me that He is there. He is taking care of our needs even down to the basic functions of life like toilet paper when we received about an 8 month supply. Terra was asked what we need on her Facebook or blog page and her response was toilet paper. So many people brought us toilet paper. It was awesome but I still refused to give Him the credit. I have made conscious decisions to not give him the credit for sustaining my family. I know in my heart that He is providing and yet I would get frustrated for all the Mito issues he abandoned us on. Twisted, I know. I struggle today to give him thanks for all he has given us but I think I am making a turn. Maybe this letter/blog post is a big step towards the right direction.

I have been bitter towards Terra. (This will not be a rip on Terra section but it is only used for me to explain my feelings through this whole Mito crap and why I hate it so much). I would ask myself “Why has she been so focused on living in the world of the internet instead of me?” I know that Terra has an absolutely amazing support group on the internet and this group is probably reading this right now. Terra has been able to get support and bounce ideas off of other individuals with kids dealing with similar Mito issues as Kendall. I can now say that I am so very grateful for all of you that support Terra. I know that she would not be sane today without you. I know that Terra has essentially received her Doctorate through this experience and internet searches if only for the sole purpose of keeping Kendall alive and wanting more for her. I am thankful that I have been able to steadily work through this and don’t feel bitterness towards Terra anymore. So, Terra I am sorry for this. I know that it is a selfish struggle that I need to continue to eliminate. Besides, how can I be upset with her for seeking the support of others when I myself am not supporting her because I keep everything held tight inside of me?

I always feel that we are separated as a family. Of course, a major part of that separation is my job and all the travel that I do for work. However, I am so thankful for a job. I am thankful that I have very understanding bosses within my department and work in a company that values the needs of my family. It sucks getting that 3:00 am phone call or text from Terra saying there is an issue but I am so grateful that I can pack my bags and hit the road for home with no fear or questions about my job. I am a very scheduled person. If my schedule is broken by some outside force (Mito) I cannot stand it. It frustrates me. However, again I am thankful that I have a position that I make my own schedule for about 45 of the 52 weeks per year. I am able to adjust as needed. Again, I am thankful for that but hate that Mito has caused the need for it.

I am angry at this situation that Mito has caused for my four beautiful amazing daughters. They don’t get to experience life as what many would consider “normal life”. We do not get to take what most would consider family fun “Clark Griswold vacations”. First of all we don’t have the financial capabilities to take said vacations due to all of the costs associated with Mito care but in reality we just have so much junk that we would have to prepare for us to take all the medical crap associated with Mito care. We have been able to take some one night get-a-ways to downtown Chicago but even with that we basically have a mini traveling hospital that we must tote around with us. I hate that most of my family’s get away mini vacations don’t always include the six of us. Too often it is me coming home from travel for the week and Terra going away for some mommy-big-sisters get away time. I am grateful that they can go away together as the big girls need that alone time with mommy. I just hate that this is how they have to get it due to all the Mito issues.

The girls also have to deal with a plethora of people coming in and out of our house. From some horror stories that I have heard we are very blessed to have the team of nurses and therapists that we do have. We get along with them, they have been in place consistently and I get the feeling that they genuinely care about Kendall and my family. In my opinion our two nurses go above and beyond almost daily when they come to our house. Not only do they take care of Kendall but have helped out in so many other ways with the older girls. Although I am grateful that we have care for Kendall it saddens me that the other girls have to deal with all these other people in the house. It saddens me because although they may not think anything of all the faces in our house this Mito crap as created a “non-normal” environment for them to live in and deal with.

I hurt for Kealey Grace and the daily struggle she has dealing with all too often being the mommy to the other girls because I am on the road for work and mommy has no choice but to address Mito issues. Kealey Grace is such a strong kid, she amazes me how well she can keep herself together. Unfortunately I am seeing more and more that she is like her father when it comes to holding in her emotions and then lashing out at her sisters in frustration.

Karissa Lynn is such a lover. She clings to any kind of love that someone shows her. I only hope that I can provide the love and support that she needs so that some day she doesn’t cling to others that are not good for her. I hate that I too often am so wiped out from travel during the week and trying to take over Mito care on the weekends that beautiful Karissa is forgotten. For this I have been making a conscious effort to make sure she is loved at home but I know she still craves it from me.

Kaylen Hope, Hurricane Kaylen as we call her. I love this little bunch of spitfire more than I think I could ever express. Her stubbornness and individualism reminds me of Kealey Grace x 10. The big girls have always had the outlet of school to get away from the constant reminder of Mito issues. However Kaylen has never had that luxury. She all too often plays second fiddle to the daily dealing with Mito issues. This is just her reality because she is home. I am so happy for her that she started preschool this year. She can make her own friends and forge her own way in this world. However, I have told her many times that she can’t grow up. She’s not allowed to get any older than 4 yrs old.

Even though I have listed out so many things that I hate from the affects of Mito there have been some very positive and downright amazing things that have come out of our situation. As a family we have seen the unbelievable outpouring from so many. So many people that I don’t think Terra and myself could even list out half of the people who have so unselfishly helped us in tangible ways. We have had so many provide us with meals, financial support, babysitting etc. We had a baseball team take us under their wings and do a fundraiser for someone who they had never met or seen. Others have helped coordinate care for our older girls. We have also had so many pray on a continual basis for Kendall, for Terra, for me individually and as a family. I cannot express my appreciation enough for all who have kept us in their prayers. You all are the reasons that my family is still together and I cannot thank you enough. Being the provider for the family I need continued prayer to realize that I need to lean on the True Provider and stop relying on myself for everything. Nothing is mine any ways, everything I have has been given to me to be looked after, if only for a short while. It is not mine to keep.

I have also been able to witness what I will call an absolute miracle. Kendall Quinn is such an amazing person. Not just an amazing kid but an amazing person. Her strength, spirit, and personality are unmatched. Her smile is contagious. She may be in tremendous pain but will still flash that smile. It absolutely melts my heart and brings me to tears at the same time.

Terra and I have learned to take the simple things in life as precious moments. Although sometimes the craziness of four daughters running around the house and all the craziness that ensues drives me to a breaking point I have to take a step back and rejoice that I still have four daughters creating the very chaos driving me into insanity. Reality is that we don’t know how long that will last.

I have learned that next to Kendall Quinn who is the toughest person I know, Terra is right behind her. Terra’s strength and Wonder Woman type qualities (minus the cool outfit) are mystifying to me. I will make this statement that I’m sure so many of you will agree with. Kendall Quinn is alive today for two reasons. First and foremost our Lord is not finished with her yet here on earth. Although I do not know, nor may I ever know what his plan is, I am immensely grateful He has given us this beautiful gift that is Kendall Quinn. Second, Kendall is still here today because of her mothers’ strength to never take no for an answer, to never let a Doctor except mediocrity for Kendall Quinn. Terra I thank you for your strength. You amaze me.

So this has been slightly longer than the paragraph that Terra had asked for but realistically how can I explain all of this in one paragraph. Actually someone please check on Terra, she may have fainted knowing I actual had this much thought going on in my head. I guess my usual self of hiding my emotions and feelings has finally been tipped of kilter, well at least for a short while. I ask for your continued prayers my Kendall, Terra, the other girls and myself. As I work through my struggles in dealings with Mito Issues your prayers may just help me make the conscious decision to accept the help that He has been so wanting for me to accept for so long.

Thank you for letting me tell you my story.

To all of my Ladies; Terra, Kealey Grace, Karissa Lynn, Kaylen Hope and Kendall Quinn… I love you all so very much. Thank you for still loving me through all of this.

Benj.

A Great Day.

Today I was the guest poster over at Dana’s blog – you remember Dana, she blogged here on Tuesday about her son “Looking Great”!

October 20 2009 017 Anyways – today I wrote a post for her blog about what it takes for Kendall (and similarly so many other mito kids) to actually HAVE one of those “great days”. thank you to the many of you who stopped by to read her post here, now go on over to her blog and check out my post and some of her other awesome posts – both about mito awareness week as well as her other myriad topics! She’s got a fun little place over there!

 

Check it out HERE.

 

Anyways – today I had hoped to be able to have a little vlog of the girls telling you about their thoughts on mito and mito awareness week, but our videographer was…not having a very organized day!

I do have some more thoughts I want to get out here, but I wanted to direct those of you who don’t get the notifications on FB to know where I am today!

Go on over to Dana’s, drop me a line over there, or drop me a line here. Or do both! cause you know how much i LOVE COMMENTS! AND EMAILS!

 

thanks for checking in on us – check back later for you nite owls and west coasters on THIS post as I will probably update/edit it later.

and then your regularly scheduled random rambling from me, here on my humble abode, will return tomorrow!

Here is a list of all the blog posts from this week in case you missed any!

Sunday – What is Mito?

Monday – Journey to a Diagnosis

Tuesday – He Looks Great – guest post by Dana

Wednesday – Give and Take

 

Thanks for checking on us!

 

terra.

Give and Take.

Our days are filled with the “give and take” nature of  a sometimes invisible disease process. Most days when blog1 things are going well, there is nothing to give, and it takes nothing from us. Well, almost nothing. Or maybe its just that we’ve already given so much, we don’t notice what is being taken. But other days, the days i want to throw things at the TV when smarmy, smug pregnant women tell me that THEY are going to give THEIR BABIES the best things in life by breastfeeding/using similac/no enfamil/cloth diapering/huggies/drinking V8/taking 5hour Energy, or the commercials about chubby babies playing and cooing happily make me want to break down in tears for the fact that I was too busy pleading with God for my child’s life to enjoy that roly-poly time most babies get – on those days, it feels like so much has been taken from us. From her. From my friends who have had to bury their children.

There is a song that is sung in our church as part of our worship service. It is called “Blessed Be The Name of the Lord”. It is based on a Psalm, and is basically the Psalmists way of saying – no matter what happens, i’m going to CHOOSE to praise you, God.

(lyrics by Matt Redman)

Blessed Be Your Name
In the land that is plentiful
Where Your streams of abundance flow
Blessed be Your name

Blessed Be Your name
When I'm found in the desert place
Though I walk through the wilderness
Blessed Be Your name

Every blessing You pour out
I'll turn back to praise
When the darkness closes in, Lord
Still I will say

Blessed be the name of the Lord
Blessed be Your name
Blessed be the name of the Lord
Blessed be Your glorious name

Blessed be Your name
When the sun's shining down on me
When the world's 'all as it should be'
Blessed be Your name

Blessed be Your name
On the road marked with suffering
Though there's pain in the offering
Blessed be Your name

Every blessing You pour out
I'll turn back to praise
When the darkness closes in, Lord
Still I will say

 

You give and take away You give and take away 
My heart will choose to say Lord, blessed be Your name
That last part is so hard to sing most Sundays. it always has been. Because if i can’t sing it, then i am 
just doing a bunch of lipservice.
it’s not REAL. it’s just a religion, not a relationship.
 But that’s probably a whole other post. What I am trying to say here and now is this – our lives have been
 affected by mito in ways that are hard to
even put in to words.

Somedays I feel “angry” at all that has been “taken” from my baby, or from our family:
The ability to eat and enjoy so many foods without pain (and usually other medical issues that result 
if she tries!)
The ability to play outside like a regular kid.
the ability to go places as a family (she usually stays home with her nurse to not over-stress her body)
the ability to swim, take a bath, splash with her sisters on a warm day in the sprinklers.
the ability to go climb into her own big girl toddler bed.
the ability to have a body free from tubes and wires and sensory overloads and 
not-quite-up-to-par functions of body systems
the ability to live a pain free, happy go lucky life free of hospitals and specialists offices

I could really get going on the things that i feel are “taken”. It would be SO EASY some days to give 
in to the bitterness that takes root so easily in the soul when life doesn’t go “just like we hoped it would”.
But the truth of the matter is, that it has not taken HER from us, so what really do we have to complain about.
And the truth of it is, that all of that, up there, is not the focus of our lives, 
and i never want it to be what takes front and center stage here on my blog.
With the bad comes the good.
So what have we been given, what blessings have been bestowed upon us “because” of what Kendall’s 
diagnosis brings us…
We have been given an insight into what “priorities” really means.
We have watched our daughter exhibit a strength and bravery at her young age that would make ANY 
parent proud.
we have been the recipients of people giving of their time and money to help us stay together 
in times and places it seemed impossible.
we have witnessed the GOODNESS of human nature – and let me tell you – that is a priceless gift indeed.
we have stood on the edge of a dark cliff, looked the abyss in the eye, and said 
“not today – you’re not takin her today”.
 the incredible amount of strength and confidence you get from being able to be in that place is 
an indescribable feeling. Most people will never know what i’m talking about. And for them i am both
incredibly happy, and incredibly sad.
We have seen people exhibit “friendship” and “walking beside us” in ways that are so much deeper 
than surfacey lip-service.
We have learned what it means to be “rich in happiness”, and how extremely healing 
a good deep belly laugh can be.
We have learned the meaning of “simple joys” – of rejoicing in the mundane, the miniscule, the miraculous
 – a first smile after an illness, a new word whose timing
is surprising, a mess made that represents a milestone met.
We have learned that CHOOSING TO BE GRATEFUL is to choose life.
We are learning that making that choice is rarely easy, but it is always worth it.


I read a blog post recently (go HERE to read it for yourself), and it struck such a chord of resonance
in my soul. It speaks of a word the Israelites would use after they had been given a blessing
 (seriously just go read her blog,
she explains it WAY better than i ever could!) How often can we say “Dayenu” – it would have been enough. 
when I think about this struggle
between “give and take away”, I realize that it is not quite right. We were never promised that we would
be “given” anything in life. No one ever sat me down and promised me that i would have four beautiful
baby girls and they would all be happy roly poly Gerber babies free of problems and brattiness.
No one promised that our lives would be without storms and cold nites and hard days and really crappy stuff.
No one has ever promised me that Kendall would be healed completely and live a totally normal life.
No one ever promised us that WE would love from one sunrise to the next.
And so in our good times and our bad times, we must learn to say “Dayenu” – It is enough.
It is enough that we are given this breath, this moment, this day.
It is enough that we have what we have been blessed with by our God.
It is enough that we have memories, treasured times, blessings, life.
God is good when our prayers are answered, and HE IS GOOD when they are not.
It is not mine to say “you give me this, you can’t take that away”.

And so I will continue to sing, with tears in my eyes, and the words faltering out, 
and with a slight tinge of jaw clenching because
these words are not easy to get out:
you give and take away.
you give and take away.
my heart will choose to say,
Lord, blessed be your name.



Terra.


Many many thanks to Lore, author of the blog referenced above, for her post that inspired this post today.

Mito:Journey to a Diagnosis

This post might be a hard one to get out. Not because of the emotion of it, although that plays a part. But more because it brings up so many questions than answers. That’s always been kind of the nature of it with Kendall, though. More questions than answers.

We are often asked when we first knew something was “wrong” with Kendall (and people usually ask it with a sense of trepidation – like they hesitate to say that something is “wrong” with someone. but it’s ok, there’s something wrong with all of us – i am not personally offended if people word the question like this – because i’d rather have you ASK the question than make assumptions!). But the answer is that we knew from the second she was born. And if we are honest with ourselves, we knew it from a few weeks out from her birth, when i started having lots of problems with the pregnancy, things we just chalked up to my age, to back-to-back pregnancies, to my own chronic fatigue/fibromyalgia problems. Essentially what it seems like was happening was that as her energy needs were becoming more and more overwhelming, my body was failing to keep up with the production.

She was born “blue”, essentially not breathing very well at all. The midwife whisked her away image to begin administering oxygen and sternal rubs, and the NICU was called. You can read more about what happened at birth here. So from the start,  she was having issues in many areas. Her list of “diagnoses” started growing in the NICU before we even brought her home. But we did eventually bring her home. The doctors kept telling us that “any day now she will wake up and she will know how to breathe and eat on her own then”. So we sat and slowly slowly slowly bumped that oxygen regulator down and we shoveled 15cc’s of formula into her with a bottle every four hours (and maybe ignored a little too much the fact that at least 10 of those cc’s were coming back up as puke right away) – but whatever – we just wanted her HOME with us! We wanted to believe what the doctors were saying, that she was just going to wake up and outgrow it and life would be back to NORMAL.

But it wasn’t normal.

We still had to take her for weekly weight checks, where we got a different doctor every time, telling us something different every time. At her 2 month checkup she got her first set of shots and this is when literally all hell broke loose. she started puking and pooping (at the same time) literally almost nonstop for the next ten days. She was pretty much not ever awake during this time, but again, we were told this was normal and fine and we were just still experiencing ‘NICU anxiety”. When this episode started was when i first began to have my suspicions that something more was really going on – as they had never been able to pinpoint her issues in the NICU beyond “transient tachypnea of newborn” – essentially she breathes too fast cause she doesn’t know any better. This did not explain her temperature dysregulation, her inability to gain weight (she actually lost a lot of weight in the NICU), her apparent infection with some unidentified bug (immune issues?), or the fact that she failed the newborn screening three times. We were not told any of this information at that time, only found out after we had left the hospital. They told us that her newborn screen fails were nothing to worry about, and i still to this day do not know WHAT or HOW she failed them. Three years later, its kind of a moot point. She’s been tested for basically everything under the sun that can be tested for, and we get the same results: Abnormal but not diagnostic. Meaning, she has a lot of characteristics of a lot of different metabolic diseases, but does not contain that particular gene defect in her chromosomes.

By 13 weeks old, 3 weeks after the psychotic vaccine issue, she had a wicked cold. She was rushed from the ped’s office girls summer 09 049to the nearest hospital by ambulance, and that was when i knew this stuff was about to get REAL. She somehow survived that nite. I still to this day do not know how. My mom showed me a picture a few weeks ago of a blue skinned, glassy eyed, scrawny little baby, and my heart stopped as i realized that was MY baby, that nite, breathing what i can only imagine is something a step or two above “the death rattle”. If I ever doubted that God has something in store for this little girl – I look back at those pictures, and I think of that nite, and I know HE saved her, for some other purpose or reason. After narrowly surviving that nite, she was transferred to a regional childrens center an hour and a half from our home where she was fully worked up by a team of about 7 specialists in every specialty you can think of.

This is where we first heard the words “possible mitochondrial disease”. A neurologist at this hospital was quite concerned with the fact that Kendall was so extremely hypotonic (floppy), and that she had issues systemically. There was much talk of doing a muscle biopsy at that time (considered to be the gold standard for diagnosing many muscular and metabolic myopathies), but no one felt she was strong enough to survive such a procedure. She had gone in to the hospital with RSV, but ended up with bilateral pneumonia, which quickly turned into sepsis (systemic blood infection). She needed to have a PICC line placed for extra fluids and heavy duty medications, and even sending her to be sedated for that procedure was one fraught with anxiety and worry that she could not withstand the demands placed on her body.

But she did! And in spite of a few more hospitalizations throughout that horridly long spring, she finally began to do well with a specialized formula pumped into her stomach via a small tube we passed through her  kqNG nose and into her tummy (hopefully. sometimes we’d get it into her lungs and have to start the whole horrid process over again.) To say she was “doing well” is somewhat misleading in itself though too, because there were still many many nights when one or both of us would just lay awake with our hands on her chest, making sure she was still breathing. “Doing well” meant simply that she was not in the hospital being septic as we had seen her get in February, so we considered this an improvement.

She ended up getting a g-tube placed and a Nissen wrap performed to try to help force her stomach to work and absorb foods. When even these measures failed to “fix” many of her scary symptoms, we were referred to Childrens Hospital of WI in Milwaukee where they had a motility center to assess more of what was going on with her. From the first ten minutes we spent with our GI (gastrointestinal) doctor up there, we knew we were in the right place. The first few vials of bloodwork that he ordered began immediately to show issues with her metabolic system, and at our second appointment when we had JUST gotten some results back, he paged genetics from the room and told them to clear their schedule NOW and see this kid. Again we were schlepping back and forth from specialist to specialist, getting some pieces of the puzzle here, missing this or that symptom there, trying this that and the other thing in an effort to make her better. But after a few weeks, a lot more tests, and TONS more vials of blood sent to heck and gone, we finally got more confirmation that she had a presumed mitochondrial disease, etiology unknown, shortly after her first birthday.

august 2009 044She was continuing to struggle with meeting milestones developmentally, and at 9 months after yet another immunization trial, she lost head control and many of the skills she had worked so hard to gain in the previous four months of intensive therapy.

She  also at this time had extremely high levels of a metabolic byproduct called “methylmalonic acid”, leading her geneticists  to believe that she had a particular form of mito known as “SUCLA2”. We began to combat the acid levels that were building up in her system with daily injections of very potent vitamin B12 (read: the amount most adults would need in one month, we were giving to her every day, in her little teeny 9 lb body). Genetic testing came back negative for the known genes that cause SUCLA2, but she did have an “as yet unidentified” variant on one of the genes. Still though, this was not enough for our geneticist to say he had found her form of mito disease, and by then we were well into having more pressing issues to deal with than what we would call kendall’s disease!

She had her G-tube switched to a G-J (gastrojejunal) tube to feed her formula directly into her intestines when she was 14 months old, an idea which her team had suggested for about 5 months before I could finally wrap my mind around it. It just seemed like such a step backwards. But it was a huge help to Kendall that august 2009 049 was undeniable. She struggled with lots of illness that winter, at one point sleeping upwards of 21 hours a day, with a body temp that never rose above 94 degrees. Clearly things weren’t all rosy still! But we were still getting little to no direction as to WHAT was going on – just a lot of shoulder shrugs and "try to keep her warm!” I am sure if we were closer to her team, things may have been different on that end. But we’re a stubborn bunch around here, and we were bound and determined to just fix her with our love, so we didn’t run off to the doc every time she did something that scared us to death, and we just learned to try to help her deal as best as she could, as best as WE could.

So even though she had had the prerequisite “3 or more body systems involved in ways that do not otherwise seem to relate to each other” since birth, and even though we had LOTS and LOTS of bloodwork and even a muscle biopsy that all pointed towards a metabolic mitochondrial disease, and even though pretty much everyone on her medical team (roughly 12 docs, 5 therapists, a handful of nurses) assumed it was mito – we still felt like we needed to make sure no other stone was unturned, make sure we weren’t missing something that might be fixable.  So in August of 2010, Kendall and I rode a series of little prop planes across the midwest to the Childrens Hospital of Pittsburgh, to meet with one of the few known “mito experts” in the country. Kendall was admitted for a week of intense testing, and at the end of the week, while we had a few other red herrings we COULD try to chase down, the consensus was that Kendall’s mitochondria were indeed dysfunctional, and were responsibleDSC_0148 for causing her multiple issues. And just in case I had any doubt about this diagnosis, or needed time “for it to sink in”, Kendall started having a massive metabolic breakdown literally within minutes of walking out of that hospital that week, and ended up sustaining damage to her GI tract that she still has not recovered from. Sure she got better, and eventually did come home from the hospital after that episode, and even managed to have a GREAT fall season last year. But shortly after her 2nd birthday, she got RSV AGAIN, and began a series of hospitalizations that ended finally this past June of 2011. {On Wednesday I will blogging more about what life looks like for Kendall these days, how she is affected currently, in case you are wondering about that!}

to say that our road to a diagnosis has been anything but easy is quite an understatement.

in fact, i know of very few people for whom the diagnostic journey was “easy” or “straightforward” in any way. Getting news of a diagnosis of mitochondrial disease is always bittersweet – sweet in that there is finally, blessedly an answer or a REASON for all the issues that so many parents watch their children suffer through; bitter because this diagnosis brings with it no comfort.

Mitochondrial disease is currently without treatment, save for a wish and a prayer attempt at slowing down the progression of mitochondrial failure with a “cocktail” of vitamins and supplements aimed at helping the body complete the metabolic cycle of energy production. Since this cocktail is experimental at best, and contains “vitamins”, insurances do not cover it. The amount of these vitamins and supplements required to see any effect is so ginourmous that it is cost ineffective to purchase them for many families, especially considering that the only feasible way to consume the cocktail is to have it “compounded” – the hundreds of pills required on a daily basis are crushed into liquid form. $$$$!!!  Not only is there no known treatment or cure for this disease, it is often fatal at a young age. This is a hard stat to type because it is the part of Kendall’s diagnosis that I do my hardest to ignore. 80% of the kids who are diagnosed in the first two years of life with mito do not live to see their tenth birthdays. It is my sincerest hope that this statistic somehow magically changes over the next 7 years, for the much improved. This year,though, an exciting announcement was made that some scientists have created a drug that is being proven to drastically reduce symptoms for some of the most affected mito patients, most notably those with severe neurologic implications. This is SO exciting! It is still in the testing phase, and is not available to “the general mitochondrial population” yet, but it is a glimmer of HOPE. It makes me so sad that this news comes too late for so many of the angels we have lost this year, and last, and the year before that…But as the mito logo says – kendallBHOPE. ENERGY. LIFE.  These are the things we hold on to.

Mitochondrial disease is in itself an enigma. there is little rhyme or reason as to when or how it will strike. Some children have relatively mild symptoms right up to puberty, and then cannot survive the stress of the demands puberty puts on a body. Others are hit hard and heavy in the early years, but then seemingly find their stride and even have hope of making some slight improvements. Still others tend to stay stagnant in their symptoms, wherever on the spectrum they happen to fall. But one thing is certain – mito is unpredictable. It keeps you on your toes. The next fever your child gets could lead to major regressions and a lengthy hospitalization, or it could be just a blip on the screen. It is a state of constantly waiting for the other shoe to drop. That was a very hard adjustment for Ben and I as Kendall’s parents – not knowing when or how to react to some of her “issues”. And we still don’t “know” – but I think we’ve come to a kind of understanding with Kendall’s body, with her disease, however or whatever it will eventually be named. We will do our best to support her body in the areas it is weak, and her body will do its best to try to stay even keeled. We have no guarantees…

but does anybody really?

Our path to a diagnosis has not been an easy one. But we are not alone. On a long hard journey, having companions is always best. And above all – we are blessed. oh so very very blessed. Our daughters diagnosis makes us appreciate every sunset, every sunrise, every laugh, every crazy moment. We see life in a way differently than so many people get to experience – seeing the joy in the mundane, the smiles in the rain, the blessings in the midst of the storms. It’s kind of like getting a room on the 87th floor of a building with no elevator when you really would have preferred the 2nd or 3rd floor. It’s redonkulously hard some days, but if you just sit back and take a look at the view…oh its breathtaking. Gorgeous beyond description. Sit back and take it in.terratalkingdays1

I could ramble on this topic for another 37 pages. If you’ve made it this far, thanks.

To all of you on a similar journey – my heart is with you. It’s a journey no one wants to be on, but the rewards that come with the challenges – incomparable.

To our friends who have gone ahead of us and laid the path that we can pick out – thank you is not enough.

To those who walk beside us and with us on a day in and day out basis – keep on keepin’ on.

 

terra.

What is Mitochondrial Disease?

In honor of Mitochondrial Disease Awareness Week, I thought I’d start off with a post about what exactly IS mitochondrial disease (“mito”)? And truthfully – it’s a question that some of the best and brightest minds in the medical world have a hard time answering.

At the base of the science behind mito is this explanation from the United Mitochondrial Disease Foundation website:

Mitochondrial diseases result from failures of the mitochondria, specialized compartments present in every cell of the body except red blood cells. Mitochondria are responsible for creating more than 90% of the energy needed by the body to sustain life and support growth. When they fail, less and less energy is generated within the cell. Cell injury and even cell death follow. If this process is repeated throughout the body, whole systems begin to fail, and the life of the person in whom this is happening is severely compromised. The disease primarily affects children, but adult onset is becoming more and more common.
Diseases of the mitochondria appear to cause the most damage to cells of the brain, heart, liver, skeletal muscles, kidney and the endocrine and respiratory systems.

And they have this cute drawing to explain it:

image

Got it now?

 

Neither do I!

As we have been on this journey to figure out a diagnosis for Kendall for almost 3 years now, I have made it somewhat my mission in life to UNDERSTAND more of what “mito disease” is and means. Because quite often not even our doctors can understand or explain it to us. So here is my somewhat more simplified (and perhaps not quite so scientific) version of what exactly mito is and does.

Essentially, every cell in your body has a little engine called a “mitochondria”. The cells know what they are supposed to do because the mitochondria help map/direct the cells – so the liver cells know to be a liver, your eyes know how to be eyes, your gut knows how to digest food, etc. (again – very simple, not scientific – stay with me!) Those mitochondria help make the ENERGY for those cells to do their job, by taking the byproducts of the food you eat, and the oxygen you breathe, go through a super complex metabolic process, something about the Krebs cycle, and bam – they create a little packet of energy, the cell does its job, and you live your life.

BUT – if you have a mitochondrial disease, something is wrong with the directions that that mitochondria is giving out. Maybe it has the wrong info encoded into it because of a genetic defect, or maybe it is just super inefficient at its job of creating energy packets. But basically, that fun little cycle of metabolism is messed up, and not enough energy packets are made. No bueno.

You know how when you are getting sick, or ARE sick, or are getting over being sick – and you just feel kind of “blah”? It’s probably a big combo of things (maybe you puked nonstop, or just couldn’t breathe real well, or some other unpleasant combo) – but the point is – your body is diverting a TON of energy to your immune system to help you GET BETTER from that sickness, so you may not have the energy you typically do to go running or exercising, or even get off the couch and make dinner. Your mitochondria in all those areas are probably still all working, but their energy packets are being “borrowed” by your immune system so it can activate your T-cells and WBC’s etc etc etc.

In a person with mito, that “blah” feeling is kind of “as good as it gets”. Their bodies are in a constant state of needing to “borrow” energy packets because they just can’t make enough energy to go around. This is part of why there can be such a wide variation of symptoms and severity among mito patients – because some people are able to create more energy to go around, and others are just never going to be able to get on top of their energy stores.

I tell most people who we are first meeting and describing kendall’s issues to to think of it like this:

Imagine that you have to pay $5000 in bills every month (i know – i can only think in round numbers, go with it). But – in spite of working 3 jobs and pinching every penny you can, you can only come up with about $3500. You have a choice – you can either shuffle a lot of bills, and pay everyone a little bit, enough to keep the collectors at bay but never enough to actually PAY your bills, or you can choose to only pay certain important ones. Now say you get sick – you can only work maybe ONE job, and your income is only enough to cover the mortgage that month. Everyone else suffers, but the mortgage is paid. Eventually all those late and overdue bills are going to catch up with you though, unless you can figure out some huge way to pay them all off.

this is how Kendall’s body is affected by mito.

k4hosp2i Every day, she is only able to produce a certain amount of energy. Prior to being able to get on top of some of her biggest issues (respiratory, metabolic, and GI – tummy), she was in a constant state of robbing Peter to pay Paul, and even Peter was going bankrupt real fast. Her body is not able to produce enough energy to sustain ALL of the demands her body has. She does not appear to be able to utilize oxygen and food in an appropriate manner to complete the metabolic cycle necessary to produce an efficient “energy packet”. This seems to affect her in just about every system – and we will get more into HOW it affects Kendall this week, but for now, that’s the basic summary of WHAT mito means.

It is a disease of energy metabolism.

So little is known about HOW this disease occurs. Scientists and doctors only began to identify  mito as a unified disease process (vs just a bunch of “really bad luck” hitting some kids multiple times over) in the late 80’s-early ‘90’s. Progress on the research and understanding of mito has been painstakingly slow as due to the breakdown of mitochondria, other diseases can be more “fitting” than this little known and little understood disease process. There is now much talk of connecting diseases like Parkinsons, Alzheimers, Autism and many many many cases of SIDS to an underlying mitochondrial disorder. There are only a handful of doctors around our country who are able to properly diagnose and “assess” mitochondrial diseases in many kids, and their labs are sadly underfunded, and these doctors are terribly overworked. Of the nearly 3000 genes responsible for mitochondrial function, scientists have thus far been able to identify (breakdown and understand and “map out”) roughly 300 of them. Those 300 genes have been linked to about 20 of what we call the “named” mitochondrial disorders – they are initials aimed at naming the prominent symptoms of that disease – MERRF, MELAS, MNGIE, NARP, KSS, and a handful of others. Sadly in med school, most doctors have one class that quickly goes over ALL of those diseases, and again, its mostly only the named ones. THOSE diseases are quite rare in the general population – but unfortunately mitochondrial disease as a whole is NOT rare. It is believed that just as many children are born every year with mito as are born with cystic fibrosis. Mito disease affects just as many kids in varying degrees as pediatric cancers.

And yet it is so unheard of, so unknown how to treat it…

There are so many amazing families out there living with mitochondrial disease, families like us who are having to forge our way ahead in this crazy world, educate doctors out of our own home-grown knowledge, trying to find that balance between doing anything and everything to try to MAKE THIS BETTER for our kids and letting them just be kids in their own ways. I hope that over this week you will enjoy hearing some other stories, learning more about what mito IS and what it looks like, and mostly that you will walk away being able to share with someone else that mito is real, and it needs some REAL attention by people who can help us DO something to cure this horrible disease!

As always – if you have any questions about mito or about anything I may write this week – PLEASE feel free to email me!  terra@2sisphotos.com

 

Thanks so much for stopping by, for reading, for being interested in learning more about mitochondrial disease. Your interest is part of the cure. Your desire to understand how mito affects Kendall’s life, and our lives, gives it a validity and a meaning that some days is hard to figure out. I cannot thank you all enough~ from the bottom of my heart!

 

terra.

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